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Title: Hereditary cerebral hemorrhage with amyloidosis in patients of Dutch origin is related to Alzheimer disease

Abstract

Hereditary cerebral hemorrhage with amyloidosis in Dutch patients is an autosomal dominant form of vascular amyloidosis restricted to the leptomeninges and cerebral cortex. Clinically the disease is characterized by cerebral hemorrhages leading to an early death. Immunohistochemical studies of five patients revealed that the vascular amyloid deposits reacted intensely with an antiserum raised against a synthetic peptide homologous to the Alzheimer disease-related ..beta..-protein. Silver stain-positive, senile plaque-like structures were also labeled by the antiserum, yet these lesions lacked the dense amyloid cores present in typical plaques of Alzheimer disease. No neurofibrillary tangles were present. Amyloid fibrils were purified from the leptomeningeal vessels of one patient who clinically had no signs of dementia. The protein had a molecular weight of approx. 4000 and its partial amino acid sequence to position 21 showed homology to the ..beta..-protein of Alzheimer disease and Down syndrome. These results suggest that hereditary cerebral hemorrhage with amyloidosis of Dutch origin is pathogenetically related to Alzheimer disease and support the concept that the initial amyloid deposition in this disorder occurs in the vessel walls before damaging the brain parenchyma. Thus, deposition of ..beta..-protein in brain tissue seems to be related to a spectrum of diseases involving vascular syndromes,more » progressive dementia, or both.« less

Authors:
; ; ; ; ;
Publication Date:
Research Org.:
New York Univ. Medical Center, NY (USA)
OSTI Identifier:
5209443
Resource Type:
Journal Article
Journal Name:
Proc. Natl. Acad. Sci. U.S.A.; (United States)
Additional Journal Information:
Journal Volume: 84:16
Country of Publication:
United States
Language:
English
Subject:
59 BASIC BIOLOGICAL SCIENCES; CEREBRUM; CYTOCHEMISTRY; HEREDITARY DISEASES; PATHOGENESIS; NERVOUS SYSTEM DISEASES; PROTEINS; AMINO ACID SEQUENCE; HEMORRHAGE; IMMUNOLOGY; MENINGES; PATIENTS; VASCULAR DISEASES; BIOCHEMISTRY; BODY; BRAIN; CENTRAL NERVOUS SYSTEM; CHEMISTRY; DISEASES; MEMBRANES; MOLECULAR STRUCTURE; NERVOUS SYSTEM; ORGANIC COMPOUNDS; ORGANS; PATHOLOGICAL CHANGES; SYMPTOMS; 550201* - Biochemistry- Tracer Techniques; 551001 - Physiological Systems- Tracer Techniques

Citation Formats

van Duinen, S G, Castano, E M, Prelli, F, Bots, G T.A.B., Luyendijk, W, and Frangione, B. Hereditary cerebral hemorrhage with amyloidosis in patients of Dutch origin is related to Alzheimer disease. United States: N. p., 1987. Web. doi:10.1073/pnas.84.16.5991.
van Duinen, S G, Castano, E M, Prelli, F, Bots, G T.A.B., Luyendijk, W, & Frangione, B. Hereditary cerebral hemorrhage with amyloidosis in patients of Dutch origin is related to Alzheimer disease. United States. https://doi.org/10.1073/pnas.84.16.5991
van Duinen, S G, Castano, E M, Prelli, F, Bots, G T.A.B., Luyendijk, W, and Frangione, B. 1987. "Hereditary cerebral hemorrhage with amyloidosis in patients of Dutch origin is related to Alzheimer disease". United States. https://doi.org/10.1073/pnas.84.16.5991.
@article{osti_5209443,
title = {Hereditary cerebral hemorrhage with amyloidosis in patients of Dutch origin is related to Alzheimer disease},
author = {van Duinen, S G and Castano, E M and Prelli, F and Bots, G T.A.B. and Luyendijk, W and Frangione, B},
abstractNote = {Hereditary cerebral hemorrhage with amyloidosis in Dutch patients is an autosomal dominant form of vascular amyloidosis restricted to the leptomeninges and cerebral cortex. Clinically the disease is characterized by cerebral hemorrhages leading to an early death. Immunohistochemical studies of five patients revealed that the vascular amyloid deposits reacted intensely with an antiserum raised against a synthetic peptide homologous to the Alzheimer disease-related ..beta..-protein. Silver stain-positive, senile plaque-like structures were also labeled by the antiserum, yet these lesions lacked the dense amyloid cores present in typical plaques of Alzheimer disease. No neurofibrillary tangles were present. Amyloid fibrils were purified from the leptomeningeal vessels of one patient who clinically had no signs of dementia. The protein had a molecular weight of approx. 4000 and its partial amino acid sequence to position 21 showed homology to the ..beta..-protein of Alzheimer disease and Down syndrome. These results suggest that hereditary cerebral hemorrhage with amyloidosis of Dutch origin is pathogenetically related to Alzheimer disease and support the concept that the initial amyloid deposition in this disorder occurs in the vessel walls before damaging the brain parenchyma. Thus, deposition of ..beta..-protein in brain tissue seems to be related to a spectrum of diseases involving vascular syndromes, progressive dementia, or both.},
doi = {10.1073/pnas.84.16.5991},
url = {https://www.osti.gov/biblio/5209443}, journal = {Proc. Natl. Acad. Sci. U.S.A.; (United States)},
number = ,
volume = 84:16,
place = {United States},
year = {Sat Aug 01 00:00:00 EDT 1987},
month = {Sat Aug 01 00:00:00 EDT 1987}
}